KLIPPELYA-FEYLYA DISEASE

From Big Medical Encyclopedia

KLIPPELYA-FEYLYA DISEASE (M. Klippel, fr. neurologist, 1858 — 1942; A. Feil, fr. neurologist, sort. in 1884; synonym Klippel's syndrome — Feylya) — inborn deformation of cervicothoracic department of a backbone with reduction of number of vertebrae and their union in one bone array. The disease is for the first time described as a syndrome by Klippel and Feyl in 1912. Arises at one on 42 thousand newborns at an identical ratio of floors. A constant triad To. — T. — short neck, low border of hair behind, restriction of mobility of the head.

Merge of several cervical and verkhnegrudny vertebrae in a uniform conglomerate is characteristic of a disease. Number of cervical vertebrae no more than 4, and they are often underdeveloped.

Outward of the child at To. — T. it is characteristic: the head sits as if on shoulders, the chin touches a thorax. Ear lobes are often extended and hang over the shoulder girdles. Asymmetry of the person and skull, splitting of a hard palate, deformation of teeth, existence of alate folds of a neck, an atrophy of muscles of a shoulder girdle, a nevus pigmentosus of skin are quite often noted. Characteristic symptoms are a bone form wrynecks (see), scoliosis (see), kyphosis (see). In 40% of cases high standing of shovels (Shprengel's deformation) is observed. Other deformations of a musculoskeletal system are observed. In 10% of cases malformations of various bodies take place.

Fig. 1. The tomogram of a neck in a side projection at Klippel's disease — Feylya: characteristic deformation (merge of vertebrae) of cervical department of a backbone (it is specified by an arrow).
Fig. 2. The roentgenogram of cervicothoracic department of a backbone in a direct back projection at Klippel's disease — Feylya: bone merge of vertebrae in nizhnesheyny and verkhnegrudny departments (it is specified by an arrow), sharp rapprochement of upper three edges, cervical department of a backbone it is shortened.

Diagnosis of a disease is based on a wedge, symptoms with an obligatory rentgenol, inspection of cervical and verkhnegrudny departments of a backbone in back and side projections. At very short neck on the x-ray image of cervical department of a backbone in a direct back projection shadows of bones of a facial skull and an occipital bone are imposed. In similar cases the roentgenogram and furthermore the tomogram in a side projection (fig. 1), as a rule, reveal all characteristic details and allow to make the correct diagnosis with confidence.

The basic rentgenol, a sign To. — T. deformation cervical, and sometimes and verkhnegrudny department of a backbone (fig. 2), expressed in merge of several bodies of vertebrae is owing to what the vertical size of a neck is sharply shortened in comparison with norm. Along with it chaotic ossification and merge of arches of vertebrae and their shoots, and also not fusion of arches of vertebrae can be observed. Bone merge of bodies of vertebrae can sometimes be incomplete and between them the narrow strips of an enlightenment corresponding to underdeveloped cartilaginous intervertebral disks are visible. At rather seldom observed complications To. — T., the connected with a prelum back and a brain, special techniques rentgenol. researches — vertebralny angiography (see), miyelografiya (see) — allow to establish an actual reason and character of a complication.

At differentiation with a tubercular spondylitis it must be kept in mind that for To. — T. deformation of vertebrae without signs of inflammatory and destructive changes is characteristic.

At children apply the conservative method of treatment directed to strengthening of the general state, the prevention of secondary deformations. At small efficiency of conservative treatment and bystry progressing of deformations of soft tissues the operational treatment performed usually at teenage age is shown. Rough deformations of a backbone at this disease cannot be corrected, but the deformations connected with changes in soft tissues, existence of cervical edges, curvatures of clavicles in a varying degree give in to correction. Operational treatment is carried out in several steps. Main types of operations are skin, the myoplasty, corrective bone operations. Cosmetic effect of operational treatment favorable.



Bibliography Dyachenko V. A. Anomalies of development of a backbone in X-ray anatomic lighting, M., 1949, bibliogr.; Kazmin A. I. and Fomicheva E. U. To a question of operational treatment of a disease of Klippel — Feylya, Ortop, and travmat., JSfc 10, page 67, 1974; P and N about in N. A., and N-told And. 3. and Mo with to ach ev and K. A. Radiodiagnosis in pediatrics, page 37, M., 1972, bibliogr.; Reynberg S.A. Radiodiagnosis of diseases of bones and joints, book 2, page 180, M., 1964; T and-ger I. L. and D I am the p e of N to about V. A. Radiodiagnosis of diseases of a backbone, page 65, M., 1971, bibliogr.; Gunderson of Page H. and. lake of The Klippel — Feil syndrome, Medicine (Baltimore), v.# 46, p. 491, 1967, bibliogr.; Hensinger R. N., Lang J. E. a. Mac E w e n G. D. Klippel — Feil syndrome, J. Bone Jt Surg., v. 56-A, p. 1246, 1974; Klippel M. et Feil A. Un cas d’ab - • sence des vertebres cervicales avec cage tho-Tacique remontant jusqu’& la base du crane (cage thoracique cervicale), Nouv. Iconogr. Salpet., t. 25, p. 223, 1912; Nora J. J., Cohen G. M. a. Maxwell A. Klippel — Feil syndrome with congenital heart disease, Amer. J. Dis. Child., v. 102, p. 564, 1961.


S. L. Nikitina; L. M. Freydin (rents.).

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